Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ewing's family tumor (EFT) is a rare pediatric tumor of unclear origin that occurs in bone and soft tissue. Specific chromosomal translocations found in EFT cause EWS to fuse to a subset of ets transcription factor genes (ETS), generating chimeric EWS/ETS proteins. These proteins are believed to play a crucial role in the onset and progression of EFT. However, the mechanisms responsible for the EWS/ETS-mediated onset remain unclear. Here we report the establishment of a tetracycline-controlled EWS/ETS-inducible system in human bone marrow-derived mesenchymal progenitor cells (MPCs). Ectopic expression of both EWS/FLI1 and EWS/ERG proteins resulted in a dramatic change of morphology, i.e., from a mesenchymal spindle shape to a small round-to-polygonal cell, one of the characteristics of EFT. EWS/ETS also induced immunophenotypic changes in MPCs, including the disappearance of the mesenchyme-positive markers CD10 and CD13 and the up-regulation of the EFT-positive markers CD54, CD99, CD117, and CD271. Furthermore, a prominent shift from the gene expression profile of MPCs to that of EFT was observed in the presence of EWS/ETS. Together with the observation that EWS/ETS enhances the ability of cells to invade Matrigel, these results suggest that EWS/ETS proteins contribute to alterations of cellular features and confer an EFT-like phenotype to human MPCs.
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PMID:Inducible expression of chimeric EWS/ETS proteins confers Ewing's family tumor-like phenotypes to human mesenchymal progenitor cells. 1821 50

Multipotent mesenchymal stromal cells (MSCs) can be isolated from bone marrow or peripheral blood. To identify phenotypical and functional differences between MSCs derived from these sources, the human bone marrow-derived, fibroblast-like cell line L87/4 was compared with the peripheral blood-derived, fibroblast-like cell line V54/2. Both cell lines expressed similar levels of SH3+, CD45(-), CD68(-), CD133(-), and HLA-DR(-). The bone marrow-derived cells expressed higher surface levels of CD105, CD10, and CD117 and preferentially expressed alkaline phosphatase, glutathione S-transferase P, and cofilin-1. The peripheral blood-derived line showed a higher number of CD34+/CD105+ double-positive and side population (SP) cells. The results demonstrate the more multipotent, yet quiescent, stromal phenotype of bone marrow MSCs, whereas MSCs isolated from the circulation display more hematopoietic-lineage characteristics. Importantly, potential marker genes that distinguish the two stages of MSCs are defined.
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PMID:Alkaline phosphatase, glutathione-S-transferase-P, and cofilin-1 distinguish multipotent mesenchymal stromal cell lines derived from the bone marrow versus peripheral blood. 1823 66

Papillary renal cell carcinoma (PRCC) can display extensive areas of solid and non-papillary architecture and extensive areas with oncocytic cytoplasm. Eleven oncocytic renal cell neoplasms (ORCN) with histopathological features posing a diagnostic problem between renal cell carcinoma (RCC) with oncocytic features and renal oncocytoma (RO) were identified. The neoplasms were well circumscribed or encapsulated tumors with solid and diffuse growth pattern. Very occasional papillae were seen in four and tumoral necrosis in two of 11. Six ORCN displayed a CD117+/progesterone receptor (PR)+ immunophenotype (feature shared by RO) and five tumors displayed a CD117-/PR- immunophenotype (feature shared by RCC). The CD117-/PR- ORCN also displayed alpha-methylacyl-coenzyme A racemase and RCC antigen reactivity as well as varying reactivity for cytokeratin 7, vimentin and CD10 (features of oncocytic PRCC). These five cases had tumor sizes ranging from 1 to 6 cm. Two patients in the latter group developed progression of the disease with metastases. In conclusion, oncocytic PRCC with solid architecture is a rare type of RCC. The carcinoma often poses differential diagnostic problems with RO and has similar immunohistochemical properties to the common type of PRCC. Cytogenetic and molecular studies have not been performed yet for this variant of RCC.
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PMID:Oncocytic papillary renal cell carcinoma with solid architecture: mimic of renal oncocytoma. 1825 79

We report a case of pediatric acute megakaryocytic leukemia (AMKL) showing 48,XX,+21,+21 as a sole acquired cytogenetic abnormality without the mutation of GATA1 gene. A physical examination showed a phenotypically normal female. Bone marrow findings showed diffuse infiltration of leukemic blasts having scanty cytoplasm with budding blebs and prominent nucleoli, which were negative for myeloperoxide (MPO) stain, Sudan black B stain and periodic acid-Schiff stain. Immunophenotyping of leukemic cells revealed positive expression of CD34, CD13, CD33, CD117, CD41, CD61, CD7 and negative expression of TdT, anti-MPO, CD64, CD56, CD2, CD3, CD5, CD10, CD19, CD20 and CD22. A fluorescence in situ hybridization analysis showed four distinct AML1 signals in 284 of 300 interphase nuclei. The entire six exons of the GATA1 gene (7757bp) were directly sequenced. We could not find any mutations, including known polymorphisms, which are known to be involved in transient myeloproliferative disorder and acute megakaryocytic leukemia of Down syndrome. After achieving complete remission, the tetrasomy 21 disappeared.
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PMID:Tetrasomy 21 as a sole acquired abnormality without GATA1 gene mutation in pediatric acute megakaryoblastic leukemia: a case report and review of the literature. 1837 39

In this case report we present a 44-year-old woman with a 2-weekhistory of vaginal bleeding. Gynaecological examination revealed the presence of a polypoid neoformation in the endometrial cavity with a maximum diameter of 4 cm. Histological analysis showed a classic leiomyoma infiltrated by a second monomorphic, highly undifferentiated neoplasia. Immunohistochemical analysis revealed a negative reaction for cytokeratin, CD10, inhibin, CD99, CD20, CD3, TdT and CD34, and positivity for CD45, MPO, CD68 and CD117. A diagnosis of myeloid sarcoma in myometrial leiomyoma was made. The following days the patient showed the onset of an acute myeloid leukaemia M5a. Forty days after diagnosis the patient died for complications related to immunodeficiency caused by therapy. Especially when a common antibody panel reveals negativity for epithelial, mesenchymal and lymphoid markers, this case underlines the importance of considering myeloid sarcoma in differential diagnosis of undifferentiated tumours arising in an extramedullary site in order to avoid errors and permit optimal therapeutic management.
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PMID:[Myeloid sarcoma in uterine leiomyoma]. 1868 22

The clinicopathologic and immunohistochemical features of 69 pediatric examples of infantile digital fibroma/fibromatosis (IDF) were analyzed. Thirty males, 26 females, and 1 child (sex unstated) ranging from newborn to 120 months of age (median, 12 mo) manifested 74 lesions (5 identified in follow-up) involving the toe or finger (n=71) and the hand or foot (n=3). Tumors ranged in size from 3 to 35 (median, 10) mm. All but 4 study members presented with a solitary lesion. Metachronous IDFs developed in 7 patients within 17 to 82 months. Microscopically, a cytologically bland, fibroproliferative lesion was observed forming a dome-shaped/polypoid nodule directly beneath the epidermis and invading dermal adnexa. Mitotic figures per 20 high-powered fields ranged from 0 to 7 (median, 1). Paranuclear cytoplasmic inclusions were identified in 57 tumors. Tumor cells immunohistochemically expressed calponin (11 of 11 tumors), desmin (9/9), alpha-smooth muscle actin (11/11), CD99 (11/11), CD117 (6/8), heavy caldesmon (2/11 and scattered cytoplasmic inclusions in 4 tumors), CD10 (1/9), nuclear beta-catenin (2/11), and CD34 (1/11), but not muscle actin (HUC1-1), keratins, estrogen/progesterone receptor proteins, or activated caspase-3. Twenty-eight of 38 patients (74%) experienced recurrent/persistent disease (single in 22; multiple in 6) (median, 4 mo after surgery). One recurrent tumor spontaneously regressed and the size of another remained unchanged for almost 17 years before reexcision. All 23 patients with >5 years follow-up are currently disease free (median disease-free interval, 23 y). Minor postoperative functional/cosmetic complaints were reported in 47%. No patient with adequate clinical data developed the digitocutaneous dysplasia syndrome or a conventional fibromatosis, or relayed a family history of IDF/conventional fibromatosis. Our results indicate that IDF is a unique myofibroblastic process separable from conventional fibromatoses and from histologic mimics. Conservative excision or observation after biopsy (with additional surgery employed as necessary) are recommended treatment options.
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PMID:Infantile digital fibroma/fibromatosis: a clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up. 1883 Jan 28

Renal oncocytoma (RO) is a characteristic benign renal tumor. The existence of malignant RO is controversial and anecdotal, partly due to a lack of specific markers for RO. With recent advances in immunohistochemistry, RO can be distinguished from other renal neoplasms with routine stains and with the aid of immunostaining. We report two cases of renal neoplasms with similar histopathological appearances. They were characterized by oncocytic cytoplasm, numerous intra-cytoplasmic vacuoles, uniform round to oval hyperchromatic nuclei with remarkably thick nuclear membranes and prominent nucleoli. The tumor cells were closely packed and disposed in an alveolar pattern. The neoplastic cells were diffusely reactive for CD117 and progesterone receptor, and diffusely or focally reactive for cytokeratin AE1/AE3, and focally reactive for cytokeratin 7, CD10, and racemase. The cells were non-reactive for renal cell carcinoma (RCC) antigen, vimentin, S100, and neuroendocrine markers. One tumor showed lymph node metastasis. Due to the remarkable cytological atypia, lymph node metastasis, and similar immunological features of RO, these two tumors likely represent a distinct subtype of RCC related to RO.
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PMID:Oncocytic renal cell carcinoma with immunohistochemical properties of renal oncocytoma. 1894 37

We report on a case of a 30-year-old male with acute B-lymphoblastic leukemia (B-ALL) with immunophenotype CD19(+), CD22(+), CD20(+), CD10(+), with aberrant expression of CD13 and CD117, and IgH gene rearrangements. Three months after treatment with GMALL-2003 and Ida/FLAG protocols bone marrow showed predominance of blasts with myeloid morphology and phenotype MPO(+), CD13(+), CD33(+), CD64(+), CD15(+), CD56(+), EVI-1 gene overexpression and lack of IgH rearrangements. The case is the first report of a very early emergence of myeloid leukemia during the induction treatment for B-ALL in an adult patient. Different pathogenetic mechanisms are discussed - clonal evolution or selection, lineage switch or development of a de novo or therapy-induced leukemia.
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PMID:Very early onset of an acute myeloid leukemia in an adult patient with B-cell lymphoblastic leukemia. 1914 72

This study was aimed to investigate the immunologic characteristics of refractory anemia with excess blasts-II (RAEB-II) which belongs to a new subtype of World Health Organization (WHO) classification of myelodysplastic syndrome (MDS) and to screen out the independent immunologic prognostic factors of MDS. 35 cases of adult patients with de novo MDS were investigated. The immunofluorescent analysis by multiparameter flow cytometry was performed at the double gating of CD45/SSC to determine the immunophenotype of MDS cells in all cases. All patients were followed up. 47 cases of acute myeloid leukemia (AML) M1, 51 cases of AML-M(2) and 38 cases of acute lymphocytic leukemia (ALL) were selected as control. Software SPSS 13.0 was applied to analyze all the related data. The results showed that the positive expression rate of HLA-DR in RAEB-II was 100%, which was high in sensitivity and specificity. CD13 (94.74%), CD33 (84.21%) and CD117 (78.95%) were also highly expressed in RAEB-II. CD13 in RAEB-II was significantly higher than that in refractory cytopenia with or without multilineage dysplasia (RA/RCMD) (p < 0.01) and REAB-I (p < 0.05); CD33, CD117 (p < 0.05) and stem cell antigen CD34 (p < 0.01) in RAEB-II were significantly higher than that in RCMD (p < 0.01), but no statistically significant difference was found as compared with RAEB-I (p > 0.05). Compared with AML-M(1) and AML-M(2), no significant difference of CD13 and CD117 in RAEB-II was found (p > 0.05). CD33 (p < 0.01) and CD34 (p < 0.05) were significantly lower than that in AML-M(1), but no significant difference was found as compared with AML-M(2) (p > 0.05); CD15 (p < 0.01) and CD11b (p < 0.05) was significantly lower than that in M(2), but no significant difference was found as compared with AML-M(1) (p > 0.05); MPO was significantly lower than that in AML-M(1) and M(2) (p < 0.05); HLA-DR was significantly higher than that on AML-M(2) (p < 0.05), but no significant difference was found as compared with AML-M(1) (p > 0.05). RAEB-II did not express CD2, CD3, CD5 and CD8 (positive rate 0%, p < 0.01) when compared with T-ALL; CD4 (p < 0.05) and CD7 (p < 0.01) were significantly lower than that in T-ALL. RAEB-II did not express CD19 and CD20 (positive rate 0%, p < 0.01) as compared with B-ALL; CD10, CD22 and cCD79a were significantly lower than that in B-ALL (p < 0.05). CD117 (p = 0.0197) and MPO (p = 0.0085) were the two prognostic immunological antigens as regards the overall survival (OS) of MDS; CD117 (p = 0.003) was the single parameter in Cox regression. It is concluded that RAEB-II expresses mainly myeloid antigen without or with little expression of lymphoid antigen. Unique individual immunophenotypic features can be detected in patients with RAEB-II. HLA-DR can be a specific parameter to distinguish the other subtypes of MDS. CD117 may be an independent prognostic immunological antigen as regards OS of MDS.
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PMID:Immunologic characteristics and prognosis of myelodysplastic syndrome new subtype: refractory anemia with excess blasts-II. 1923 59

Reported herein are seven cases of a histologically distinct oncocytic papillary renal cell carcinoma (OPRCC) with an inverted nuclear pattern. To define its prognostic significance, the clinicopathological features of OPRCC were compared to those of types 1 and 2 PRCC. The median age of the seven patients was 67 years. Grossly, tumors were well-circumscribed and small (1.2 cm +/- 0.4 cm). Microscopically, the OPRCC were composed of well-developed thin papillae, lined with a single layer of cuboidal-to-columnar oncocytic cells. The tumor cells had round-to-oval nuclei and eosinophilic granular cytoplasm, which was strongly positive for anti-mitochondrial immunostaining. The nuclei were characteristically polarized toward the surface of the papillae and contained mostly small nucleoli. The tumors had high expression of alpha-methylacyl-coenzyme A racemase, CD15, CD117, cytokeratin (CK) 7, E-cadherin, epithelial membrane antigen, MOC 31, mucin-1, vascular endothelial growth factor and vimentin, low expression of CD10 and Ki-67, and no expression of CK20. Genetically, gain of chromosomes 3p, 11q, and 17q, and loss of chromosome 4q was observed. All seven patients were alive with no recurrence or metastasis at a mean follow-up time of 37.1 +/- 23.7 months. In conclusion, OPRCC show unique pathological features with indolent clinical behavior and are more similar clinicopathologically to type 1 than to type 2 PRCC.
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PMID:Oncocytic papillary renal cell carcinoma with inverted nuclear pattern: distinct subtype with an indolent clinical course. 1926 Oct 90


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